This is caused by a genetic predisposition to an abnormality in, or lack of a sufficient number of lymphatic nodes or connectors within an extremity.


This can occur as a result of events such as surgery, radiation and trauma; conditions such as cancer, infection and chronic venous insufficiencies; or it can be self-induced.


Stage 1 (Spontaneously reversible)

There is an accumulation of protein-rich swelling in the tissues, which reduces at night. The swelling is soft and pitting (when pressed by a finger the area indents and holds the indentation), and there are few skin changes present.

Stage 2 (Spontaneously irreversible)

The swelling becomes incresingly hard and fibrotic, pitting is no longer present, infections become frequent and the skin deteriorates. Over a period of time, the limb gradually increases in size as fluids accumulate.

Stage 3 (Lymphestatic elephantiasis)

The limb becomes seriously deformed as volume increases dramatically; there is further deterioration in the skin with the almost continuous presence of infections (owing to the high presence of protein), cellulitis and, sometimes ulcers.

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